Overview
Definition:
Rheumatic fever (RF) is an acute, inflammatory, non-suppurative, multi-systemic illness that may develop as a delayed sequela of Group A beta-hemolytic streptococcal (GABHS) pharyngitis
It is characterized by a spectrum of manifestations affecting the heart, joints, brain, skin, and subcutaneous tissues.
Epidemiology:
While declining in incidence in developed countries, RF remains a significant public health problem globally, particularly in low- and middle-income countries
It typically affects children and adolescents aged 5-15 years
Recurrences are common and contribute to the development of chronic rheumatic heart disease (RHD).
Clinical Significance:
RF is a leading cause of acquired heart disease in children and young adults worldwide
Untreated or inadequately treated RF can lead to irreversible valvular damage (rheumatic heart disease), resulting in significant morbidity and mortality
Early diagnosis and appropriate management, including secondary prophylaxis, are crucial to prevent cardiac sequelae.
Clinical Presentation
Symptoms:
Fever
Joint pain and swelling, typically migratory and affecting large joints (arthritis)
Abdominal pain
Nausea and vomiting
Shortness of breath or chest pain (suggesting carditis)
Involuntary purposeless movements of the limbs, face, or trunk (chorea)
Skin rash, often a faint, non-itchy, pink rash with irregular, spreading edges (erythema marginatum)
Small, painless lumps under the skin, typically over bony prominences (subcutaneous nodules).
Signs:
Tachycardia and possibly murmurs or rubs on cardiac auscultation (suggesting carditis)
Tender, swollen, and warm joints with limited range of motion (arthritis)
Petechiae, purpura, or pallor
Specific neurological signs with chorea (e.g., "milkmaid's grip," facial grimacing)
Characteristic erythematous or nodular skin lesions.
Diagnostic Criteria:
The modified Jones criteria are used for diagnosis
Diagnosis requires evidence of a preceding GABHS infection (positive throat culture, rapid antigen detection test, or elevated anti-streptococcal antibody titers like ASO or anti-DNase B) plus either: 1) two major criteria, or 2) one major criterion and two minor criteria
Major criteria: Carditis, Polyarthritis, Chorea, Erythema Marginatum, Subcutaneous Nodules
Minor criteria: Fever, Arthralgia, Elevated acute phase reactants (ESR, CRP), Prolonged PR interval on ECG.
Diagnostic Approach
History Taking:
Detailed history of recent sore throat or scarlet fever
Any history of recurrent pharyngitis
Family history of rheumatic fever or rheumatic heart disease
Onset and progression of symptoms, particularly migratory joint pain, chest pain, shortness of breath, or neurological changes
Recent travel to endemic areas
Immunization status.
Physical Examination:
Thorough cardiac examination including auscultation for murmurs (new or changing), rubs, and gallops
assessment for cardiomegaly
Examination of joints for signs of inflammation (swelling, tenderness, erythema, warmth)
Neurological examination focusing on motor coordination and presence of choreiform movements
Skin inspection for erythema marginatum or subcutaneous nodules
Palpation of lymph nodes.
Investigations:
Throat swab for culture and sensitivity to rule out residual GABHS infection
Antistreptococcal antibody titers: Anti-streptolysin O (ASO) and Anti-DNase B are most useful for confirming recent infection, especially if the throat culture is negative
A single elevated titer is suggestive
a rising titer over 2-3 weeks is more definitive
Electrocardiogram (ECG) to assess for PR prolongation and other signs of carditis
Echocardiography to evaluate for valvular involvement (regurgitation, stenosis), pericardial effusion, and myocardial dysfunction, especially in suspected carditis
Complete blood count (CBC) to assess for leukocytosis and anemia of chronic disease
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) for evidence of inflammation
Chest X-ray if significant respiratory symptoms are present.
Differential Diagnosis:
Reactive arthritis (post-infectious arthritis)
Other causes of arthritis in children (e.g., juvenile idiopathic arthritis, septic arthritis, viral arthritis)
Other causes of chorea (e.g., Huntington's disease, Wilson's disease, systemic lupus erythematosus)
Viral pericarditis
Kawaski disease (shares some features but typically presents with different constellation of symptoms and signs, especially conjunctivitis and lymphadenopathy)
Psoriatic arthritis
Leukemia.
Management
Initial Management:
Hospitalization is recommended for patients with carditis, severe arthritis, chorea, or those unable to manage at home
Rest is crucial, especially with carditis
Eradication of any residual GABHS infection with antibiotics (e.g., Penicillin G benzathine IM or oral penicillin V for 10 days).
Medical Management:
Anti-inflammatory therapy: Salicylates (aspirin) are the mainstay for arthritis and carditis
Dosage typically 60-100 mg/kg/day divided into 4-5 doses, with the dose adjusted to achieve symptomatic relief and an ESR < 20 mm/hr
For severe carditis with heart failure, corticosteroids (e.g., prednisone 1-2 mg/kg/day) may be used cautiously, usually followed by salicylates
Aspirin is continued until symptoms subside and inflammatory markers normalize
Chorea may require specific management with sedatives (e.g., benzodiazepines) or anticonvulsants (e.g., valproate, haloperidol in severe cases) if significant, and is usually self-limiting over weeks to months.
Surgical Management:
Not typically required in the acute phase of rheumatic fever
Surgical intervention (e.g., valve repair or replacement) is reserved for managing the chronic sequelae of rheumatic heart disease (valvular stenosis or regurgitation) that develop years later.
Supportive Care:
Nutritional support
Psychological support for patients and families, especially with chorea
Monitoring of vital signs, cardiac function, and neurological status
Patient education regarding the importance of adherence to secondary prophylaxis.
Complications
Early Complications:
Carditis with heart failure, myocarditis, pericarditis
Severe polyarthritis causing significant pain and immobility
Acute rheumatic encephalopathy (sydenham chorea).
Late Complications:
Chronic rheumatic heart disease (RHD) due to progressive valvular damage, most commonly affecting the mitral and aortic valves, leading to stenosis and/or regurgitation
This can result in atrial fibrillation, pulmonary hypertension, infective endocarditis, and heart failure
Recurrent episodes of RF can exacerbate valvular damage and hasten the onset of RHD.
Prevention Strategies:
Primary prophylaxis: prompt and adequate treatment of streptococcal pharyngitis/scarlet fever with antibiotics to prevent the initial episode of RF
Secondary prophylaxis: continuous antibiotic therapy (usually long-acting benzathine penicillin G IM every 3-4 weeks) to prevent recurrent GABHS infections and subsequent episodes of RF and progression of RHD
Duration of prophylaxis depends on the severity of the initial illness, presence of carditis, and residual RHD.
Secondary Prophylaxis
Rationale:
To prevent GABHS recurrences, which can lead to new or progressive valve damage and chronic rheumatic heart disease.
Duration And Regimen:
Duration varies based on severity: 1) No carditis: 5 years or until age 21, whichever is longer
2) Carditis, no residual valve disease: 10 years or until age 21, whichever is longer
3) Carditis with residual valve disease: 10 years or until age 21, whichever is longer, often lifelong
Long-acting benzathine penicillin G 1.2 million units IM every 3-4 weeks for adults/adolescents, or 600,000 units for children < 27 kg
Alternatives include oral penicillin V or macrolides for penicillin-allergic patients (with careful consideration of cross-reactivity and compliance).
Compliance And Monitoring:
Strict adherence is paramount
Regular follow-up is essential for monitoring compliance, assessing for breakthrough infections, and clinical/echocardiographic evaluation for progression of RHD
Education of patient and family on the lifelong importance of prophylaxis.
Key Points
Exam Focus:
Jones criteria (major/minor), definition of carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules
Diagnostic value of ASO and Anti-DNase B titers
Primary vs
Secondary prophylaxis distinction
Regimens for secondary prophylaxis (Benzathine Penicillin G)
RHD as the major long-term complication.
Clinical Pearls:
Migratory arthritis is a hallmark of RF
Chorea can be the sole manifestation, appearing weeks or months after the initial infection
Echocardiography is essential to assess for subclinical carditis
Secondary prophylaxis is a critical component of long-term management and requires patient education and strict adherence.
Common Mistakes:
Inadequate treatment of streptococcal pharyngitis (leading to primary RF)
Discontinuation of secondary prophylaxis too early
Misinterpreting minor criteria as major or vice versa when applying Jones criteria
Failing to consider RF in children with unexplained arthritis, chorea, or cardiac symptoms.